We report the case of a 59-year-old female patient, presenting with pustular rash on both hands and pain in the lumbosacral part and left lower limb. A magnetic resonance imaging examination of the left leg was undertaken and the result showed that a malignant lesion with bone destruction of the left femoral shaft could not be excluded. Subsequently, bone tumor was excluded by pathological examination. Lung computed tomography scan showed patchy consolidation and cord shadow in the middle left lung. Subsequently, lung cancer was excluded by pathological examination, and the histopathological changes of lung were consistent with those of organized pneumonia. Blood tests revealed elevated C-reactive protein and erythrocyte sedimentation rate. Antinuclear antibody, rheumatoid factor, and human leukocyte antigen-B27 were unremarkable. Whole body bone scintigraphy via technetium 99m-methyl diphosphonate showed increased radionuclide uptake in the left middle femur. Based on her clinical manifestations, imaging results and bone scintigraphy, the patient was diagnosed as having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome. Loxoprofen and Tripterygium wilfordii Hook F led to impressive clinical and radiologic improvement.
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http://dx.doi.org/10.1111/1756-185X.14631 | DOI Listing |
Objective: The aim of this study was to evaluate and validate the accuracy and performance characteristics of administrative codes in diagnosing autoinflammatory syndromes (AISs).
Methods: We identified potential AIS patients from the electronic medical records at the University of Iowa Hospital and Clinics and the Stead Family Children's Hospital using a screening filter based on the 10th edition of the International Classification of Diseases (ICD-10) codes and interleukin-1 antagonists. Diagnostic criteria for adult-onset Still disease, systemic juvenile idiopathic arthritis, Behçet disease (BD), familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and chronic nonbacterial osteomyelitis (SAPHO-CNO) were reviewed for each patient.
Balkan Med J
January 2025
Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye.
Autoinflammatory bone diseases (AIBDs) constitute a recently identified subset of autoinflammatory diseases. These conditions are characterized by an exaggerated inflammatory response in the bones without any apparent etiology. Inflammatory bone lesions associated with AIBDs exhibit chronic inflammation, are typically culture-negative, and do not exhibit discernible microorganisms on histopathological examination.
View Article and Find Full Text PDFFront Immunol
December 2024
School of Life Sciences, Beijing University of Chinese Medicine, Beijing, China.
The complex interaction between the immune system and autoinflammatory disorders highlights the centrality of autoimmune mechanisms in the pathogenesis of autoinflammatory diseases. With the exploration of PSTPIP2, it has been discovered to play an inhibitory role in immune diseases, suggesting its potential utility in the research and treatment of rheumatic diseases. This review outlines the mechanisms of PSTPIP2 in chronic multifocal osteomyelitis (CMO), rheumatoid arthritis (RA), synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, liver diseases, renal diseases, pressure ulcer sepsis and diabetic obesity.
View Article and Find Full Text PDFJ Dermatolog Treat
December 2024
Department of Dermatology, The Second Hospital of Dalian Medical University, Dalian, China.
Aim: SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a rare autoimmune disorder characterized by typical cutaneous lesions (palmoplantar pustulosis and eruptive acne) and osteoarticular symptoms (aseptic osteomyelitis and joint inflammation). This study aims to evaluate the therapeutic efficacy and safety of Janus kinase 1 (JAK1) inhibitor abrocitinib in patients with SAPHO syndrome.
Methods: We presented a patient with SAPHO syndrome with accelerated disease progression who did not respond to traditional therapies.
Case Rep Rheumatol
October 2024
Division of Rheumatology, Mayo Clinic, Jacksonville 32224, Florida, USA.
SAPHO syndrome, a rare inflammatory disorder of bone, joints, and skin, is named based on the presence of synovitis, acne, pustulosis, hyperostosis, and osteitis. The hallmark of SAPHO syndrome includes osteoarticular and dermatologic manifestations, however, rarer associations with inflammatory bowel disease (particularly Crohn's disease) have been documented. The literature on the relationship between SAPHO syndrome and inflammatory bowel disease (IBD), especially ulcerative colitis (UC), remains limited.
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